Pulmonary surfactant homeostasis associated genetic abnormalities and lung diseases.
10.3760/cma.j.issn.1003-9406.2016.04.031
- Author:
Xiaojing JIANG
1
,
2
;
Xiuzhu SUN
;
Weihua DU
;
Haisheng HAO
;
Xueming ZHAO
;
Dong WANG
;
Huabin ZHU
;
Yan LIU
Author Information
1. Institute of Animal Sciences, Chinese Academy of Agricultural Science, Beijing 100193, China
2. College of Animal Science and Technology, Northwest Agricultural and Forestry University, Yangling, Shaanxi 712100, China. Email: liuyan05@caas.cn.
- Publication Type:Journal Article
- MeSH:
ATP-Binding Cassette Transporters;
genetics;
DNA-Binding Proteins;
genetics;
Homeostasis;
Humans;
Lung Diseases;
genetics;
Pulmonary Surfactant-Associated Protein C;
genetics;
Pulmonary Surfactants;
metabolism;
Transcription Factors
- From:
Chinese Journal of Medical Genetics
2016;33(4):564-568
- CountryChina
- Language:Chinese
-
Abstract:
Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis. This paper has provided a review for the functions and processes of pulmonary surfactant metabolism, as well as the connection between disorders of surfactant homeostasis genes and lung diseases.
